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1、Passage two common muscle disorders and diseases常见肌肉疾患 Muscles are subject to various disorders and diseases, some as simple as muscle soreness or cramp, some as complicated as myasthenia gravis, but here we would like to start with the description of the simple ones. 肌肉常受到各种疾患的侵扰,有的症状很轻,如肌酸痛或肌痉挛,有的

2、病可能很复杂,如重症肌无力。本文先从一些轻度的疾患谈起。 Muscle soreness is often caused by hard muscular work. in severe cases, the soreness may last up to four days. The cause of muscle soreness is not completely understood, but it probably involves damage to muscle and connective tissue. With proper exercise, the muscles an

3、d body can adapt to strenuous muscle work and greatly reduce the risk of tissue damage. 肌酸痛常因过度的肌肉劳累所致。严重时,症状可能会持续上数日。导致肌酸痛的机理至今尚不完全清楚,但可能是因为肌肉和结缔组织受到损害所致。通过适当的训练,肌肉和身体是可以适应强体力运动的,从而极大地减轻肌组织损害的危险。 Cramp. Muscles function through an amazing coordination of many elements. Occasionally, however, the no

4、rmal operation of muscles is disturbed. For example, a person may experience painful cramps of certain skeletal muscles if he or she exercises too hard or for a long time. Skeletal muscle cramps involve spastic (sudden and violent muscle contraction. No one knows exactly why such cramps occur. They

5、probably result from having too much or too little salt in the fluids surrounding muscle fibers. With proper rest and nutrition, the body can correct the problem, and cramping stops. Cramps also may develop in smooth muscle organs, such as the stomach and intestine. Doctors use heat, message, and me

6、dicines in treating cramps. 肌痉挛肌肉的作用是通过人体许多部位之间一种绝妙的协调来实现的。然而,不幸的是这种正常的协调偶尔间会被打断,举例说,一个人可能会因为运动过量或运动时间过长而骨骼肌发生肌痉挛,这是一种非常痛苦的经历。骨骼肌痉挛是突然和剧烈的肌收缩,没人确切知道为什么肌痉挛会发生。也许是因为肌纤维组织周围的盐份过多或过少而引起的。通过适当的休息和营养,身体自身会进行修复,痉挛就会停止。痉挛也可能发生在平滑肌构成的器官上,如胃和肠。医生常通过热敷、按摩和药物来治疗痉挛。 Myositis. Inflammation of the muscles may be e

7、ncountered in a variety of clinical settings. Direct invasion by bacteria, viruses, parasites and fungi may occur in the course of systemic infections or, less commonly, the muscle involvement may be primary (e.g. trichinosis. Bacterial toxins, such as those produced by Clostridium perfringens, may

8、injure muscle cells in the absence of direct bacterial invasion. Inflammatory myositis may also b e encountered in many of the so-called connective tissue diseases, most of which are believed to be immunologic in origin. One such immunologic disorder, which affects muscles predominantly, is polymyos

9、itis-dermatomyositis. 肌炎各种临床情况下都可发生肌肉的炎症。系统感染过程中可发生细菌、病毒、 寄生虫和真菌的直接侵袭。原发性肌肉感染较少见(如旋毛虫病)。在没有细菌直接侵袭的情况下,细菌毒素(如产气荚膜梭菌)亦可能损害肌肉细胞。某些结缔组织病也可能会出现感染性肌炎。这些疾患大多被认为是源于免疫性的。多发性肌炎一皮肌炎就是这样一种免疫性疾患,它主要的侵害对象是肌肉。 Besides above mentioned disorders and diseases, many other diseases may also affect skeletal muscles. Two

10、 major classes of muscle diseases are muscular atrophy diseases and myopathies. Atrophy diseases attack and damage the nervous system, including nerves that stimulate muscles. As a result, muscles progressively shrink and become weak. Amyotrophic lateral sclerosis, also called ALS- is an example of

11、an atrophy disease. Muscular weakness also occurs in myopathies. In these diseases, weakness results because the muscle itself does not function properly. Certain myopathies, such as various muscular dystrophies, are characterized by gradual wasting away of skeletal muscles. 除了上述的疾患外,可能影响骨骼肌的疾病尚有许多,

12、可分为肌萎缩和肌病两个大类。肌萎缩侵犯、损害神经系统,包括那些刺激肌肉的神经,该病直接导致的结果是肌肉持续萎缩和虚弱。肌萎缩性(脊髓)侧索硬化(亦被称为ALS就是一个典型的例子。肌虚弱也常见于肌病,在这类疾病中,肌肉本身没有正常发挥作用导致了肌肉的软弱。某些肌肉病变,如各类肌营养不良,其特点就是骨骼肌逐渐地消退萎缩下去。 Muscle Atrophy. At the outset we should distinguish between muscle atrophy and dystrophy, both of which may be associated with regressive

13、changes in muscles. Muscle atrophy, is an acquired lesion secondary to some well-defined predisposing cause; muscular dystrophy, on the other hand, refers to a variety of genetically determined primary disorders of muscles to be discussed later. 肌萎缩首先我们应该区分肌萎缩和肌营养不良。两者都与肌肉的消退性病变有 关,但肌萎缩是继发于某些明确素因性原因

14、的获得性病变,而肌营养不良则指多种原发的遗传性肌肉病变,下面将对此另有讨论 Atrophic shrinkage, death and disappearance of muscle cells occur under a variety of circumstances, some generalized and some local. Among the systemic disorders are chronic malnutrition, panhypopituitarism, SLE, dermatomyositis and advanced age, which presumabl

15、y lead to muscle atrophy on the basis of diffuse ischemia. In these disorders, entire muscles are a ffected uniformly. 全身或局部的肌细胞萎缩,死亡甚至消失在多种情况下都可出现。全身性疾患包括慢性营养不良,全垂体机能减退,全身性红斑狼疮,皮肤肌炎和衰老(衰老被认为是导致肌萎缩的原因之一,其机理是扩散性缺血)。在这些情况下,全身肌肉将不同程度的受累。 Localized muscle atrophy results from interference with the inner

16、vation and may be caused by traumatic denervation or neuromuscular disorders, such as polio, the peripheral neuritis and a variety of fortunately rare degenerative neuropathies. Obviously, the distribution of the muscle atrophy depends upon the pattern of involvement of the nerves. Whole muscles, bu

17、ndles of cells or only a single neuromuscular unit may be affected.局部性肌萎缩是由神经支配障碍所致,也可能因创伤而失去神经支配或神经肌肉疾病引起(如脊髓灰质炎,外周神经炎和各种幸好较少见的退行性神经病变)o很明显,肌萎缩的程度取决于神经受累的情况,整块肌肉、某个肌束、单个神经肌肉单位都可能受累。 Muscle dystrophy. This term refers to a group of genetically determined myopathies characterized by progressive atrop

18、hy or degeneration of increasing numbers of individual muscle cells. The histologic changes in the various types of muscular dystrophies are basically the same. However, the distribution of the affected muscles is quite distinctive. This, along with the mode of inheritance, forms the basic of the cl

19、assification discussed below. Muscular dystrophies must be distinguished from congenital myopathies, which are characterized by fairly specific distinctive morphologic changes. The pathogenesis of muscular dystrophies remains unknown. There is no lack of theories, but supporting evidence is scanty.

20、Recent evidence suggests the existence of a generalized membrane defect, which also involves cells other than myofibers. 肌营养不良本术语指以逐渐加重的肌萎缩或单个肌细胞增速退变为特点的一组遗传性肌肉病变。各种类型的肌营养不良的组织学改变基本相同,但受累肌肉的分布却完全不同。这些不同的分布和遗传模式是我们将要讨论的该病分类之基础。肌营养不良必须与先天性肌病相区别,后者呈鲜明的形态学改变。肌营养不良的发病机理尚不清楚。尽管有不少学说,但证据不足,最新的证据提示全身性的膜缺陷存在

21、,但这种缺陷不仅波及肌纤维,也涉及了细胞。 常见肌肉疾患(Passage Two 肌肉常受到各种疾患的侵扰,有的症状很轻,如肌酸痛或肌痉挛,有的病可能很复杂,如重症肌无力。本文先从一些轻度的疾患谈起。 肌酸痛常因过度的肌肉劳累所致。严重时,症状可能会持续上数日。导致肌酸痛的机理至今尚不完全清楚,但可能是因为肌肉和结缔组织受 到损害所致。通过适当的训练,肌肉和身体是可以适应强体力运动的,从而极大地减轻肌组织损害的危险。 肌痉挛肌肉的作用是通过人体许多部位之间一种绝妙的协调来实现的。然而,不幸的是这种正常的协调偶尔间会被打断,举例说,一个人可能会因为运动过量或运动时间过长而骨骼肌发生肌痉挛,这是一

22、种非常痛苦的经历。骨骼肌痉挛是突然和剧烈的肌收缩,没人确切知道为什么肌痉挛会发生。也许是因为肌纤维组织周围的盐份过多或过少而引起的。通过适当的休息和营养,身体自身会进行修复,痉挛就会停止。痉挛也可能发生在平滑肌构成的器官上,如胃和肠。医生常通过热敷、按摩和药物来治疗痉挛。 肌炎各种临床情况下都可发生肌肉的炎症。系统感染过程中可发生细菌、病毒、 寄生虫和真菌的直接侵袭。原发性肌肉感染较少见(如旋毛虫病)。在没有细菌直接侵袭的情况下,细菌毒素(如产气荚膜梭菌)亦可能损害肌肉细胞。某些结缔组织病也可能会出现感染性肌炎。这些疾患大多被认为是源于免疫性的。多发性肌炎一皮肌炎就是这样一种免疫性疾患,它主要

23、的侵害对象是肌肉。 除了上述的疾患外,可能影响骨骼肌的疾病尚有许多,可分为肌萎缩和肌病两个大类。肌萎缩侵犯、损害神经系统,包括那些刺激肌肉的神经,该病直接导致的结果是肌肉持续萎缩和虚弱。肌萎缩性(脊髓)侧索硬化(亦被称为ALS就是一个典型的例子。肌虚弱也常见于肌病,在这类疾病中,肌肉本身没有正常发挥作用导致了肌肉的软弱。某些肌肉病变,如各类肌营养不良,其特点就是骨骼肌逐渐地消退萎缩下去。 肌萎缩首先我们应该区分肌萎缩和肌营养不良。两者都与肌肉的消退性病变有 关,但肌萎缩是继发于某些明确素因性原因的获得性病变,而肌营养不良则指多种原发的遗传性肌肉病变,下面将对此另有讨论。 全身或局部的肌细胞萎缩

24、,死亡甚至消失在多种情况下都可出现。全身性疾患包括慢性营养不良,全垂体机能减退,全身性红斑狼疮,皮肤肌炎和衰老(衰老被认为是导致肌萎缩的原因之一,其机理是扩散性缺血)。在这些情况下,全身肌肉将不同程度的受累。 局部性肌萎缩是由神经支配障碍所致,也可能因创伤而失去神经支配或神经肌肉疾病引起(如脊髓灰质炎,外周神经炎和各种幸好较少见的退行性神经病变)o很明显,肌萎缩的程度取决于神经受累的情况,整块肌肉、某个肌束、单个神经肌肉单位都可能受累。 肌营养不良本术语指以逐渐加重的肌萎缩或单个肌细胞增速退变为特点的一组遗传性肌 肉病变。各种类型的肌营养不良的组织学改变基本相同,但受累肌肉的分布却完全不同。这些不同的分布和遗传模式是我们将要讨论的该病分类之基础。肌营养不良必须与先天性肌病相区别,后者呈鲜明的形态学改变。肌营养不良的发病机理尚不清楚。尽管有不少学说,但证据不足,最新的证据提示全身性的膜缺陷存在,但这种缺陷不仅波及肌纤维,也涉及了细胞。 肌营养不良传统上依据其初始受累的肌肉情况再分为几个

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